ICD-10 Code G11.6 – Leukodystrophy with vanishing white matter disease (2026): Diagnosis, Symptoms & Billing Guide
2026 ICD-10-CM Diagnosis Code G11.6 – Leukodystrophy with vanishing white matter disease
What it is
G11.6 identifies leukodystrophy with vanishing white matter disease, a rare inherited disorder that affects the brain’s white matter. It causes progressive loss of myelin, leading to neurologic decline over time.
Clinical signs
Typical findings include progressive ataxia, spasticity, cognitive decline, and episodes of worsening after stress or minor illness. Clinical features vary; refer to documentation.
When to use this code
Use G11.6 when the provider documents vanishing white matter disease or leukodystrophy with this specific diagnosis. Code it for confirmed hereditary white matter degeneration, not for nonspecific demyelinating disease or undifferentiated neurologic symptoms. Check documentation if the record only says leukodystrophy without subtype.
Do not use for
Do not use this code for multiple sclerosis, acquired demyelinating disorders, or other leukodystrophies unless vanishing white matter disease is clearly documented. If the diagnosis is uncertain, check documentation.
Coding tip
Look for the exact disease name in the assessment or problem list before assigning G11.6.